AC T00680
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ID T00680
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DT 16.12.1992 (created); ewi.
DT 09.05.2012 (updated); mkl.
CO Copyright (C), QIAGEN.
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FA Pax-3
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SY HuP2 (human); mPax-3; mPax3; MSF; PAX3.
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OS mouse, Mus musculus
OC eukaryota; animalia; metazoa; chordata; vertebrata; tetrapoda; mammalia; eutheria; rodentia; myomorpha; muridae; murinae
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GE G006531 Pax3.
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CL C0018; paired-homeo; 3.2.1.1.1.
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SF independent DNA-binding by paired and homeo domain contacting ATTA and GTTCC motifs, resp. [4];
SF variable spacing of the subsites [5];
SF paired domain induces conformational change in the DNA [4];
SF homodimerization upon DNA-binding through residues between paired and homeo domain [4];
SF altered DNA-binding or homodimerization in Splotch mutants [4];
SF Kr value to the binding domain (e5) of the CD19 promoter=1.3EXP4 [14];
SF mutation in paired domain (Splotch) reduces binding by factor 17 and can also reduce homeodomain binding activity (factor 21) [14];
SF interaction with HIRA by the use of the homeodomain [17];
SF interaction with pRB-family proteins (pRB, p107, p130) mediated by helix I and helix II of the homeodomain [18];
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CP 8.5-d embryos: mitotic cells in the ventricular zone of developing spinal chord; some regions in hindbrain, midbrain, diencephalon; 10-12-d embryos: neural crest of developing spinal ganglia, craniofacial mesectoderm, limb mesenchyme (10-11 d) [1].
CN differentiated neuronal cells [16].
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FF ectopic expression of Pax3 inhibits rat P0 G021489 and MBP G016272 promoter activities;
FF down-regulation of Pax3 with IL6RIL6 treatment may account for promoter induction [26];
FF the repression effect exerted by Pax3 on P0 promoter G021489 occurs without apparent DNA binding site and may be indirect, possibly because of protein-protein interactions between Pax3 and factors (e.g. Sox10 T08334) acting on myelin gene promoters [26];
FF activator (fused to GAL4-DNA binding domain) [6];
FF mutations may cause Splotch (pigmentation abnormalities in iris and hair, eye displacement, exencephaly, spina bifida, lack of limb musculature), e. g. G42R or point mutation causing aberrant splicing [2] [4] [10] [11];
FF expression is detected between embryonic day 8.5 and 14.5 [8] [9] [1];
FF absence of Sonic hedgehog (Shh) blocks expression of Pax-3/7 [12];
FF Pax-3 maintains neural progenitor cells undifferentiated [16];
FF interaction with pRB-family proteins (pRB, p107, p130) causes repression of transcription [18];
FF inhibition of the myogenic differentiation in cultured myoblast cells (C2C12 cells) [7];
FF necessary for migration but not differentiation of limb muscle precursors [19];
FF stimulation of c-met expression during limb muscle development [20] [13];
FF may play a critical role as a repressor in the place-dependent expression of Ng-CAM (neuron-glia cell adhesion molecule) in the nervous system [21];
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IN T02956 HIRA; mouse, Mus musculus.
IN T02971 p107; mouse, Mus musculus.
IN T01608 p130; human, Homo sapiens.
IN T02972 p130; mouse, Mus musculus.
IN T00680 Pax-3; mouse, Mus musculus.
IN T01423 pRb; mouse, Mus musculus.
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MX M00360 V$PAX3_01.
MX M00327 V$PAX3_B.
MX M00808 V$PAX_Q6.
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BS R29882.
BS R71308.
BS R71314.
BS R22917.
BS R22918.
BS R22924.
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DR TRANSPATH: MO000010721.
DR PATHODB: MT000032.
DR PATHODB: MT000035.
DR UniProtKB: P24610;
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RN [1]; RE0002795.
RX PUBMED: 2022185.
RA Goulding M. D., Chalepakis G., Deutsch U., Erselius J. R., Gruss P.
RT Pax-3, a novel murine DNA binding protein expressed during early neurogenesis
RL EMBO J. 10:1135-1147 (1991).
RN [2]; RE0004215.
RX PUBMED: 8421686.
RA Epstein D. J., Vogan K. J., Trasler D. G., Gros P.
RT A mutation within intron 3 of the Pax-3 gene produces aberrantly spliced mRNA transcripts in the splotch (Sp) mouse mutant
RL Proc. Natl. Acad. Sci. USA 90:532-536 (1993).
RN [3]; RE0004244.
RX PUBMED: 8099544.
RA Maulbecker C. C., Gruss P.
RT The oncogenic potential of Pax genes
RL EMBO J. 12:2361-2367 (1993).
RN [4]; RE0004245.
RX PUBMED: 7909605.
RA Chalepakis G., Goulding M., Read A., Strachan T., Gruss P.
RT Molecular basis of splotch and Waardenburg Pax-3 mutations
RL Proc. Natl. Acad. Sci. USA 91:3685-3689 (1994).
RN [5]; RE0004246.
RX PUBMED: 8065927.
RA Chalepakis G., Wijnholds J., Gruss P.
RT Pax-3 - DNA interaction: flexibility in the DNA binding and induction of DNA conformational changes by paired domains
RL Nucleic Acids Res. 22:3131-3137 (1994).
RN [6]; RE0004247.
RX PUBMED: 7809114.
RA Chalepakis G., Jones F. S., Edelman G. M., Gruss P.
RT Pax-3 contains domains for transcription activation and transcription inhibition
RL Proc. Natl. Acad. Sci. USA 91:12745-12749 (1994).
RN [7]; RE0004248.
RX PUBMED: 7744814.
RA Epstein J. A., Lam P., Jepeal L., Maas R. L., Shapiro D. N.
RT Pax3 inhibits myogenic differentiation of cultured myoblast cells
RL J. Biol. Chem. 270:11719-11722 (1995).
RN [8]; RE0004249.
RX PUBMED: 8162858.
RA Bober E., Franz T., Arnold H. H., Gruss P., Tremblay P.
RT Pax-3 is required for the development of limb muscles: a possible role for the migration of dermomyotomal muscle progenitor cells
RL Development 120:603-612 (1994).
RN [9]; RE0004250.
RX PUBMED: 7600971.
RA Goulding M., Lumsden A., Paquette A. J.
RT Regulation of Pax-3 expression in the dermomyotome and its role in muscle development
RL Development 120:957-971 (1994).
RN [10]; RE0004253.
RX PUBMED: 1682057.
RA Epstein D. J., Vekemans M., Gros P.
RT Splotch (Sp2H), a mutation affecting development of the mouse neural tube, shows a deletion within the paired homeodomain of Pax-3
RL Cell 67:767-774 (1991).
RN [11]; RE0004254.
RX PUBMED: 8406487.
RA Vogan K. J., Epstein D. J., Trasler D. G., Gros P.
RT The splotch-delayed (Spd) mouse mutant carries a point mutation within the paired box of the Pax-3 gene
RL Genomics 17:364-369 (1993).
RN [12]; RE0006702.
RX PUBMED: 8929535.
RA Ericson J., Morton S., Kawakami A., Roelink H., Jessell T. M.
RT Two critical periods of Sonic Hedgehog signaling required for the specification of motor neuron identity
RL Cell 87:661-673 (1996).
RN [13]; RE0007031.
RX PUBMED: 8633043.
RA Epstein J. A., Shapiro D. N., Cheng J., Lam P. Y. P., Maas R. L.
RT Pax3 modulates expression of the c-Met receptor during limb muscle development.
RL Proc. Natl. Acad. Sci. USA 93:4213-4218 (1996).
RN [14]; RE0010761.
RX PUBMED: 7731966.
RA Underhill D. A., Vogan K. J., Gros P.
RT Analysis of the mouse Splotch-delayed mutation indicates that the Pax-3 paired domain can influence homeodomain DNA-binding activity
RL Proc. Natl. Acad. Sci. USA 92:3692-3696 (1995).
RN [15]; RE0014013.
RX PUBMED: 7557441.
RA Chalepakis G., Gruss P.
RT Identification of DNA recognition sequences for the Pax3 paired domain
RL Gene 162:267-270 (1995).
RN [16]; RE0014025.
RX PUBMED: 9475182.
RA Reeves F. C., Fredericks W. J., Rauscher F. J. 3rd, Lillycrop K. A.
RT The DNA binding activity of the paired box transcription factor Pax-3 is rapidly downregulated during neuronal cell differentiation
RL FEBS Lett. 422:118-122 (1998).
RN [17]; RE0014075.
RX PUBMED: 9731536.
RA Magnaghi P., Roberts C., Lorain S., Lipinski M., Scambler P. J.
RT HIRA, a mammalian homologue of Saccharomyces cerevisiae transcriptional co-repressors, interacts with Pax3
RL Nat. Genet. 20:74-77 (1998).
RN [18]; RE0014085.
RX PUBMED: 9464541.
RA Wiggan O., Taniguchi-Sidle A., Hamel P. A.
RT Interaction of the pRB-family proteins with factors containing paired-like homeodomains
RL Oncogene 16:227-236 (1998).
RN [19]; RE0014110.
RX PUBMED: 8631247.
RA Daston G., Lamar E., Olivier M., Goulding M.
RT Pax-3 is necessary for migration but not differentiation of limb muscle precursors in the mouse
RL Development 122:1017-1027 (1996).
RN [20]; RE0014112.
RX PUBMED: 8681797.
RA Yang X. M., Vogan K., Gros P., Park M.
RT Expression of the met receptor tyrosine kinase in muscle progenitor cells in somites and limbs is absent in Splotch mice
RL Development 122:2163-2171 (1996).
RN [21]; RE0015185.
RX PUBMED: 7545667.
RA Kallunki P., Jenkinson S., Edelman G. M., Jones F. S.
RT Silencer elements modulate the expression of the gene for the neuron-glia cell adhesion molecule, Ng-CAM
RL J. Biol. Chem. 270:21291-21298 (1995).
RN [22]; RE0024480.
RX PUBMED: 10480898.
RA Galibert M. D., Yavuzer U., Dexter T. J., Goding C. R.
RT Pax3 and regulation of the melanocyte-specific tyrosinase-related protein-1 promoter.
RL J. Biol. Chem. 274:26894-26900 (1999).
RN [23]; RE0053016.
RX PUBMED: 16300735.
RA Murakami M., Tominaga J., Makita R., Uchijima Y., Kurihara Y., Nakagawa O., Asano T., Kurihara H.
RT Transcriptional activity of Pax3 is co-activated by TAZ.
RL Biochem. Biophys. Res. Commun. 339:533-539 (2006).
RN [24]; RE0066940.
RX PUBMED: 17662948.
RA Boutet S. C., Disatnik M. H., Chan L. S., Iori K., Rando T. A.
RT Regulation of Pax3 by proteasomal degradation of monoubiquitinated protein in skeletal muscle progenitors.
RL Cell 130:349-362 (2007).
RN [25]; RE0066970.
RX PUBMED: 18644785.
RA Farin H. F., Mansouri A., Petry M., Kispert A.
RT T-box protein Tbx18 interacts with the paired box protein Pax3 in the development of the paraxial mesoderm.
RL J. Biol. Chem. 283:25372-25380 (2008).
RN [26]; RE0035625.
RX PUBMED: 12643284.
RA Slutsky S. G., Kamaraju A. K., Levy A. M., Chebath J., Revel M.
RT Activation of myelin genes during transdifferentiation from melanoma to glial cell phenotype.
RL J. Biol. Chem. 278:8960-8968 (2003).
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